Date on Master's Thesis/Doctoral Dissertation

12-2020

Document Type

Doctoral Dissertation

Degree Name

Ph. D.

Department

Psychological and Brain Sciences

Degree Program

Experimental Psychology, PhD

Committee Chair

Mervis, Carolyn

Committee Co-Chair (if applicable)

Pani, John

Committee Member

Pani, John

Committee Member

Cashon, Cara

Committee Member

Lyle, Keith

Committee Member

Dove, Guy

Author's Keywords

Williams syndrome; 7q11.23 syndrome; pragmatic communication; language ability

Abstract

The present project examined the language abilities of children with two reciprocal neurogenetic disorders: Williams syndrome (WS), which is caused by a hemideletion of 26 - 28 genes on chromosome 7q11.23, and 7q11.23 duplication syndrome (Dup7), which results from an extra copy of the same 26 - 28 genes. Appraising the language of children with WS and Dup7 helps in understanding the communication difficulties they encounter. There were three research goals. The first was to determine the overall level of language and communication ability as measured by the Children’s Communication Checklist-2 (CCC-2; Bishop, 2006) for each syndrome, relative to chronological age expectations. The second was to determine the pattern of relative strengths and weaknesses in language form and content, pragmatic communication, and autism-related behaviors for each syndrome. The final goal was to determine the concurrent predictors of pragmatic communication ability for each syndrome. The sample consisted of 247 children with WS (126 females, 121 males) aged 6.01 – 15.50 years (M = 10.57 years, SD = 2.96) and 56 children (28 females, 28 males) with Dup7 aged 6.08 – 15.29 years (M = 10.33 years, SD = 2.82). Findings indicated that although there was considerable variability in overall language and communication ability, more than 60% of the children in each group met the CCC-2 criterion for language impairment. For most children, performance on the Language Form and Content scales was similar to performance on the Pragmatic Communication scales, a pattern characteristic of children in the general population. Each syndrome demonstrated a pattern of relative strengths and weaknesses on the CCC-2 scales. For the Language Form and Content scales, children with WS evidenced a significant relative weakness on the Coherence scale in comparison to their performance on the Speech, Syntax, and Semantics scales. In contrast, children with Dup7 evidenced a significant relative weakness on the Speech scale. Furthermore, children with Dup7 earned a significantly lower scaled score than children with WS on the Speech scale. For the Pragmatic Communication scales, children with WS demonstrated relative weaknesses on the Initiation and Use of Context scales in comparison to the Scripted Language and Nonverbal Communication scales. Children with Dup7 demonstrated a partially overlapping pattern, with relative weaknesses on the Initiation and Nonverbal Communication scales. On the Autism-related scales, children with WS demonstrated a relative strength on the Social Relations scale compared to the Interests scale. Children with Dup7 demonstrated the opposite pattern. For both syndromes, Pragmatic Communication ability was predicted by concurrent Language Form and Content ability, behavior and emotion regulation ability, and chronological age. For the WS group, sex also was a significant predictor. The effects of gene dosage on language and communication ability and implications of the findings for interventions designed to improve pragmatic communication abilities are discussed.

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